Department of Neurology, Neuroscience Center at Dartmouth, Geisel School of Medicine at Dartmouth, Hanover, NH03756,USA.
Neurobiol Dis. 2013 Jun;54:297-307. doi: 10.1016/j.nbd.2012.12.021. Epub 2013 Jan 11.
Cognitive impairment is a common comorbidity in pediatric epilepsy that can severely affect quality of life. In many cases, antiepileptic treatments fail to improve cognition. Therefore, a fundamental question is whether underlying brain abnormalities may contribute to cognitive impairment through mechanisms independent of seizures. Here, we examined the possible effects on cognition of Nav1.1 down-regulation, a sodium channel principally involved in Dravet syndrome but also implicated in other cognitive disorders, including autism and Alzheimer's disease. Using an siRNA approach to knockdown Nav1.1 selectively in the basal forebrain region, we were able to target a learning and memory network while avoiding the generation of spontaneous seizures. We show that reduction of Nav1.1 expression in the medial septum and diagonal band of Broca leads to a dysregulation of hippocampal oscillations in association with a spatial memory deficit. We propose that the underlying etiology responsible for Dravet syndrome may directly contribute to cognitive impairment in a manner that is independent from seizures.
认知障碍是儿科癫痫的一种常见合并症,可严重影响生活质量。在许多情况下,抗癫痫治疗未能改善认知。因此,一个基本的问题是,潜在的大脑异常是否可能通过与癫痫发作无关的机制导致认知障碍。在这里,我们研究了下调 Nav1.1(主要参与德拉维特综合征,但也与包括自闭症和阿尔茨海默病在内的其他认知障碍有关)对认知的可能影响。我们使用 siRNA 方法选择性地下调基底前脑区域的 Nav1.1,从而能够靶向学习和记忆网络,同时避免自发癫痫发作的产生。我们表明,在中隔和布罗卡斜角带中的 Nav1.1 表达减少会导致海马回波的失调,与空间记忆缺陷相关。我们提出,导致德拉维特综合征的潜在病因可能以与癫痫发作无关的方式直接导致认知障碍。